Langerhans cell histiocytosis first presenting in the skin in adults: frequent association with a second haematological malignancy

Abstract

Summary Background Langerhans cell histiocytosis (LCH) in adults first presenting in the skin is rare. Guidelines for staging, treatment and follow‐up are lacking. Objectives To better define staging procedures, treatment results and clinical course in adult patients with LCH first presenting in the skin. Methods Eighteen adult patients with LCH first presenting in the skin were collected from five centres collaborating in the Dutch Cutaneous Lymphoma Group. Clinical records and (skin) biopsy specimens were reviewed and follow‐up data were obtained. A literature search on adult patients with LCH presenting in the skin was performed. Results Staging procedures showed extracutaneous disease in three of 16 patients who were adequately staged. One patient had a histologically confirmed lytic LCH bone lesion, while two patients had a myelodysplastic syndrome. During follow‐up two of 18 patients developed extracutaneous localizations of LCH. Five patients developed a second haematological malignancy, including (myelo)monocytic leukaemia (two cases), histiocytic sarcoma (one case), diffuse large B‐cell lymphoma (one case) and peripheral T‐cell lymphoma (one case). Review of the literature revealed six other adult patients with a second haematological malignancy preceding or following a diagnosis of LCH. Conclusions The results of the present study suggest an increased risk of a second haematological malignancy in adult patients with LCH presenting in the skin. Extensive staging at presentation and long‐term follow‐up are therefore warranted in such patients.

High prevalence of myeloid neoplasms in adults with non–Langerhans cell histiocytosis

“…This frequency of myeloid neoplasms is much higher than that encountered general population which ranges between 0.7 and 17.1 per 100 000 people in Europe, depending on the subtype. 24 Although prior case reports and case series [25][26][27][28][29] have noted additional hematological malignancies in patients with histiocytosis of the L group, 11 the high frequency of myeloid neoplasms in a large population of adult histiocytosis patients has not previously been recognized, likely because of the relative rarity of ECD. Nonetheless, knowledge of the presence of an associated myeloid neoplasm in ECD patients has important implications for clinical management of adult histiocytosis patients as well as the classification and biological understanding of these disorders.…”

CMML: Clinical and molecular aspects

“…CMML can also be concomitantly diagnosed with mast cell [20], histiocytic [21], and blastic plasmocytoid dendritic cell disorders [22], with clonal relationship proved or strongly suspected in most cases.…”

Multifocal Cutaneous Langerhans Cell Histiocytosis

“…Cutaneous limited LCH is a very rare variant that requires careful investigation to avoid a possible systemic involvement, and presents good resolution and excellent prognosis in cases with few lesions or necrotic lesions or with hypopigmented macules [5,17,24,27,28]. However, its follow-up becomes essential due to the possibility of secondary malignant tumors, such as acute lymphocytic leukemia, lymphomas and histiocytic sarcoma, over time [8,12,[17][18][19][20]27,29].…”

“…However, its follow-up becomes essential due to the possibility of secondary malignant tumors, such as acute lymphocytic leukemia, lymphomas and histiocytic sarcoma, over time [8,12,[17][18][19][20]27,29]. While cases of extensive involvement, especially with intertriginous or perineal lesions, require chemotherapy because of the low chances of spontaneous resolution [5].…”